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We have limited information regarding the association and implications of portal venous system (PVS) anomalies in agenesis of ductus venosus (ADV) cases. Few cases of PVS malformations have been reported during fetal life apart from ADV. Our main objective was to evaluate the prenatal diagnosis of ADV and PVS anomalies and describe the outcome of these cases, either isolated or associated We evaluated the intrahepatic vascular system regarding the presence of normal umbilical drainage and PVS characteristics in the second and third trimester of pregnancy. The associated anomalies and umbilical venous drainage were noted. Genetic counseling was proposed to all ADV cases. A detailed postnatal evaluation was performed in all live births, with six months follow-ups. Ultrasonography was performed in 3517 pregnant women. 19 cases were prenatally diagnosed: 18 ADV cases, 7 abnormal PVS cases, and 6 associations of the two anomalies. We noted an incidence of 5.1‰ and 1.9‰ for ADV and PVS anomalies, respectively. Out of the 18 ADV cases, 5 (27.7%) were isolated. Karyotyping was performed in all cases: five cases (26.3%) presented genetic anomalies, Trisomy 21 being diagnosed in 60%. PVS anomalies were found in 33.3% of the ADV cases, while ADV was present in 85.7% of the PVS anomalies. Only PPVSA was encountered when umbilical drainage was normal. All TPVSA cases were associated with extrahepatic drainage of the umbilical vein. All abnormal PVS cases, except one case whose follow-up was not possible, worsened the outcome of ADV cases. Conversely, 66.6% of ADV cases with normal PVS presented a favorable outcome. DV and PVS abnormalities were found with a higher than reported frequency. Normal DV is involved in the normal development of the PVS. Additional fetal anomalies are the best predictor for the outcome of ADV cases. The functional importance of DV may be overestimated in the classic literature and textbooks since isolated ADV apparently associates a favorable outcome. Postnatal monitoring is essential to detect the implications of portal system maldevelopment, which may represent the actual prognostic factor in isolated ADV cases. [ABSTRACT FROM AUTHOR]